IKZF1 deletions in pediatric acute lymphoblastic leukemia: still a poor prognostic marker?

Abstract

Improved personalized adjustment of primary therapy to the perceived risk of relapse by using new prognostic markers for treatment stratification may be beneficial to patients with acute lymphoblastic leukemia (ALL). Here, we review the advances that have shed light on the role of IKZF1 aberration as prognostic factor in pediatric ALL and summarize emerging concepts in this field. Continued research on the interplay of disease biology with exposure and response to treatment will be key to further improve treatment strategies.

Graphical abstract

Figure 1.

Genetic alterations of the IKZF1 gene at chromosome band 7p12.2 in pediatric ALL. Red boxes indicate the observed approximate frequencies of the different types of genetic aberrations: deletions (bottom left), gene fusions (bottom right), and somatic as well as germline single-nucleotide variants (top). The 2 intronic germline risk variants identified in genome-wide association studies are indicated by their Reference SNP cluster ID. Frequencies (percentages) of the most common specific deletions (DEL) within the group of IKZF1-deleted ALL are indicated in black. Chr, chromosome; UTR, untranslated region.